Life with The Leivas: Just the Two of Us

Monday, September 28, 2009

Just the Two of Us

Amy left this weekend and Mike is back at work so it's just Olivia and I here in Durham. It feels weird for it to be just the two of us, it's been a long time! Of course Olivia and I were in the hospital by ourselves but since we were in a hospital full of people it didn't really feel like we were by ourselves. Now it really is just the two of us and we are having fun. It took a little longer to get everything ready and get us out the door this morning. We had a really long day in clinic today. Olivia's appointment was at 9:30 this morning and then they wanted to do a chest x-ray just to make sure that everything is ok. Everyone is very impressed with how well Olivia is doing but after a 2 week course of antibiotics she still has a runny nose and a bit of a cough. I was concerned, especially going into tomorrow's surgery. The chest x-ray looks great so they were able to confirm that it's an upper respiratory issue and it won't hold up tomorrow's procedure. They did start her on a different antibiotic that has helped to clear up her runny nose in the past.

Once we finished up with everything in the blood and bone marrow clinic we were just in time to head down to our pre-op appointment for Tuesday's surgery. The anesthesiologist nurse practitioner that we met with today was great. Again I am so glad that we're here at Duke, I have complete confidence that the doctors and nurses here have more experience in dealing with kids with Sanfilippo syndrome than anywhere else. Olivia's surgery is scheduled for 7:30 am Tuesday morning. The procedure should just take an hour or so. The are replacing her triple lumen Hickman catheter with a single lumen Hickman catheter. I'm told it's a simple procedure. Keep us in your prayers and check back tomorrow for an update!

2 comments:

Wendy Leiva said...: You are all always in my prayers but I will add an extra one for tomorrow. Love, Wendy; Monday, September 28, 2009 9:01:00 PM
Brandy said...: We are constantly thinking and prayer for Olivia and your whole family. Look forward to hearing how well she did in your blog.; Tuesday, September 29, 2009 3:00:00 PM

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MPS/Sanfilippo Syndrome

In October, 2008 Olivia was disgnosed with MPS III or Sanfilippo Syndrome, type B. MPS stands for: Mucopolysaccharidoses. It is a genetic lysosomal storage disease (LSD) caused by the body's inability to produce specific enzymes. Normally, the body uses enzymes to break down and recycle materials in cells. In individuals with MPS and related diseases, the missing or insufficient enzyme prevents the proper recycling process, resulting in the storage of materials in virtually every cell of the body. As a result, cells do not perform properly and may cause progressive damage throughout the body, including the heart, bones, joints, respiratory system and central nervous system. While the disease may not be apparent at birth, signs and symptoms develop with age as more cells become damaged by the accumulation of cell materials.

Babies and young children with Sanfilippo Syndrome appear normal, but symptoms begin to appear with age as more and GAGs build up in the cells of the body. There are 3 stages to the disease. Stage 1 the child begins to lag behind peers and begins to display difficult behaviors. Stage 2 the child losing his/her language, becomes hyperactive, chews on everything, and has sleeping difficulties. Stage 3 the child slows down, becomes dependant for all mobility and loses the ability to chew/swallow. There is no treatment or cure for Sanfilippo. Life expectancy varies.